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Ehlers-Danlos syndrome

Ehlers-Danlos syndrome is a group of inherited disorders that affect your connective tissues — primarily your skin, joints and blood vessel walls. Connective tissue is a complex mixture of proteins and other substances that provides strength and elasticity to the underlying structures in your body.

People who have Ehlers-Danlos syndrome usually have overly flexible joints and stretchy, fragile skin. This can become a problem if you have a wound that requires stitches, because the skin often isn't strong enough to hold them.

A more severe form of the disorder, called vascular Ehlers-Danlos syndrome, can cause the walls of your blood vessels, intestines or uterus to rupture. If you have vascular Ehlers-Danlos syndrome, you may want to talk to a genetic counselor before starting a family.

Symptoms
Causes
Complications
Prevention

Signs and symptoms of the most common form of Ehlers-Danlos syndrome include:

Overly flexible joints. Because the connective tissue that holds joints together is looser, your joints can move far past the normal range of motion. Small joints are affected more than large joints. You might also be able to touch the tip of your nose with your tongue.

Stretchy skin. Weakened connective tissue allows your skin to stretch much more than usual. You may be able to pull a pinch of skin up away from your flesh, but it will snap right back into place when you let go. Your skin might also feel exceptionally soft and velvety.

Fragile skin. Damaged skin often doesn't heal well. For example, the stitches used to close a wound often will tear out and leave a gaping scar. These scars may look thin and crinkly.

Fatty lumps at pressure points. These small, harmless growths can occur around the knees or elbows and may show up on X-rays.

Symptom severity can vary from person to person. Some people with Ehlers-Danlos syndrome will have overly flexible joints but few or none of the skin symptoms.

Vascular Ehlers-Danlos syndrome

People who have the vascular subtype of Ehlers-Danlos syndrome often share distinctive facial features of a thin nose, thin upper lip, small earlobes and prominent eyes. They also have thin, translucent skin that bruises very easily. In fair-skinned people, the underlying blood vessels are very visible through the skin.

One of the most severe forms of the disorder, vascular Ehlers-Danlos syndrome can weaken your heart's largest artery (aorta), as well as the arteries to your kidneys and spleen. A rupture of any of these blood vessels can be fatal. The vascular subtype also can weaken the walls of the uterus or large intestines — which may also rupture.

What to expect from your doctor

Extremely loose joints, fragile or stretchy skin, and a family history of Ehlers-Danlos syndrome are often enough to make a diagnosis. Genetic tests on a sample of your blood can confirm the diagnosis in some cases and help rule out other problems.

Blood pressure. Because blood vessels are more fragile in some types of Ehlers-Danlos syndrome, your doctor may want to reduce the stress on the vessels by keeping your blood pressure low.

Physical therapy

Joints with weak connective tissue are more likely to dislocate. Exercises to strengthen the muscles around a joint can help stabilize the joint. Your physical therapist might also recommend specific braces to help prevent joint dislocations.

Surgical and other procedures

In rare cases, surgery is recommended to repair joints damaged by repeated dislocations. However, your skin and the connective tissue of the affected joint may not heal properly after the surgery.

Coping with a lifelong illness is challenging. Depending on the severity of your symptoms, you may face challenges at home, at work and in your relationships with others. Here are some suggestions that may help you cope:

Increase your knowledge. Knowing more about Ehlers-Danlos syndrome can help you take control of your condition. Find a doctor who's experienced in the management of this disorder.

Tell others. Explain your condition to family members, friends and your employer. Ask your employer about any accommodations that you feel will make you a more productive worker.

Build a support system. Cultivate relationships with family and friends who are positive and caring. It may also help to talk to a counselor or clergy member. Support groups, either online or in person, help people share common experiences and potential solutions to problems.

Helping your child cope

If you are a parent of a child with Ehlers-Danlos syndrome, consider these suggestions to help your child:

Maintain normalcy. As much as possible, treat your child like other children. Ask others — grandparents, aunts, uncles, teachers — to do the same.

Be open. Allow your child to express his or her feelings about having Ehlers-Danlos syndrome, even if it means being angry at times. Make sure your child's teachers and other caregivers know about your child's condition. Review with them appropriate caregiving skills, particularly in the event of a fall or injury.

Promote activity. Encourage your child to participate in physical activities with appropriate boundaries. Discourage contact sports while encouraging non-weight-bearing activities, such as swimming. Your child's doctor or physical therapist also may have recommendations.